Wiedemann-Beckwith syndrome and hypothyroidism
✍ Scribed by Alexander K. C. Leung
- Publisher
- Springer
- Year
- 1985
- Tongue
- English
- Weight
- 44 KB
- Volume
- 144
- Category
- Article
- ISSN
- 0340-6997
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## Abstract Beckwith–Wiedemann syndrome (BWS) is a clinically heterogeneous overgrowth syndrome associated with an increased risk for embryonal tumor development. BWS provides an ideal model system to study epigenetic mechanisms. This condition is caused by a variety of genetic or epigenetic altera
The Wiedemann-Beckwith syndrome (WBS) comprises an accumulation of multiple congenital anomalies. Exomphalos, macroglossia and gigantism are considered the most common manifestations, hence the alternative designation EMG-syndrome. The syndrome carries with it an increased risk of developing specifi
Wiedemann-Beckwith syndrome (WBS) has already been noted to have asymmetric expression in monozygous (MZ) twins. Up to now 12 sets of MZ twins with WBS have been reported (Table I). To this series we would like to add 2 more sets of female twins, both discordant for WBS. TWIN PAIR 1 This set of twi
## Abstract Monozygotic (MZ) twinning occurs with relatively high frequency in Wiedemann‐Beckwith syndrome (WBS). Ten sets of MZ twins with WBS have been reported. Nine of these have been female and in each case the twins were discordant for the WBS phenotype. The tenth set was male. They were conc
Two boys and two girls from a sibship of six, affected with the Wiedemann-Beckwith syndrome (WBS), are reported. One of the patients also had congenital hypothyroidism, an association hitherto undescribed and possibly fortuitous. Neither stigmata of WBS in other family members nor parental consangui